Laminin isoforms in development and disease.
Författare
Summary, in English
The members of the laminin family of heterotrimers are major constituents of all basement membranes, sheet-like extracellular structures, present in almost all organs. The laminins bind to cell surface receptors and thereby tightly connect the basement membrane to the adjacent cell layer. This provides for the specific basement membrane functions to stabilize cellular structures, to serve as effective physical barriers, and furthermore, to govern cell fate by inducing intracellular signalling cascades. Many different types of diseases involve basement membranes and laminins. Metastasizing solid tumors must pass through basement membranes to reach the vascular system, and various microbes and viruses enter the cells through direct interaction with laminins. Furthermore, whereas mutations in one specific laminin chain lead to a muscular disorder, mutations of other laminin chains cause skin blistering and kidney defects, respectively. This review summarizes recent progress concerning the molecular mechanisms of laminins in development and disease. The current knowledge may lead to clinical treatment of lamininopathies and may include stem-cell approaches as well as gene therapy.
Publiceringsår
2007
Språk
Engelska
Sidor
825-836
Publikation/Tidskrift/Serie
Journal of Molecular Medicine
Volym
85
Issue
8
Länkar
Dokumenttyp
Artikel i tidskrift
Förlag
Springer
Ämne
- Cell and Molecular Biology
Nyckelord
- lamininopathies
- dystroglycan
- integrin
- basement membranes
Status
Published
Forskningsgrupp
- Muscle Biology
ISBN/ISSN/Övrigt
- ISSN: 1432-1440